Treating CP for Life

If you're looking for a pattern among individuals with cerebral palsy (CP), the only one you're likely to find is that there is no pattern. Each individual presents unique challenges.

One way to understand CP is to think of it in terms of cancer, explains Ginny Paleg, PT, MS, DScPT, with Montgomery County Schools Infant and Toddler Program in Rockville, Md. CP itself is a very broad diagnosis.

Paleg says that the diagnosis is described by the limb affected, such as hemiplegia for one side or quadriplegia for all four limbs; by quality, such as spastic or hypotonia; and by movement disorder, such as ataxia and dystonia.

Cerebral palsy can result from a variety of causes. According to United Cerebral Palsy (UCP), these causes include brain injury during the birthing process, brain infections and head injury after birth.

Early signs of CP are usually apparent before 18 months of age, when children fail to reach developmental milestones, such as walking, crawling and smiling. UCP estimates that approximately 764,000 children and adults in the United States have some form of CP.

CP Defined

"A new trend is for physicians, especially neurologists, to distance themselves from the diagnosis of CP and instead name the brain anomaly," Paleg says. "This helps us so much in treatment."

For example, Paleg says that if a client has periventricular leukomalacia (PVL), then she knows the trunk will always be weak, and stabilization is a necessity to prevent the limbs from getting stiffer.

"If you have dystonia, I have to watch during puberty for a fast-developing scoliosis," she says. "If you have athetosis, I better give you another chance to learn to walk around ages 8-12, when the frontal lobe starts working as a motor planning center."

The four main types of cerebral palsy are spastic, athetoid (or dyskinetic), ataxic and mixed. Spastic cerebral palsy is the most common type, affecting 70 to 80 percent of patients, according to UCP. In this type, the muscles are stiffly and permanently contracted. Subcategories of this type describe which limbs are affected, such as spastic diplegia (both legs).

The limb description provides a clue as to how therapists will need to work with the client. "A lot of times those kids with diplegia are going to end up being higher-functioning kids," explains Mary Miles, PT, DPT, with White Bear Lake School District in Minnesota. "The kids with quadriplegia, (in which) all of their limbs are involved, they're going to end up being more involved kids that maybe will end up using wheelchairs all of their day or part of their day."
Paleg says it's important to remember that even if only one side of the body is affected, the "better side" is still affected.

With athetoid CP, uncontrolled, slow, writhing movements in the hands, feet, arms or legs - and, in some cases, the muscles of the face and tongue, causing grimacing or drooling - are present. People with athetoid CP also may have problems coordinating the muscle movements needed for speech. Problems usually exist with balance and involuntary movements, says Mark Smith, consumer research/outreach manager, Quantum Rehab, Exeter, Pa.

Ataxic cerebral palsy is a rare form that affects balance and depth perception, resulting in poor coordination; an unsteady, wide-based gait; and difficulty with quick or precise movements, such as writing or buttoning a shirt. These clients also might exhibit intention tremor. Smith explains that ataxic CP usually affects fine motor skills and visual and auditory processing.

If those classifications are confusing, it might be best to simply consider tone, Miles says. "I think what makes sense is to look at the high tone and low tone because a lot of the treatment is based on those two types."

For example, hypotonicity, or low tone, is usually present with ataxia, which looks like tremors. "Underlying all of that ataxia is really just a lot of low tone," Miles says. "A lot of those kids can be a little bit milder too (and) they end up walking and just look sloppier ... unless it's a different kind of brain injury where the ataxia is just so bad that they can't end up walking."

Mixed forms of CP combine symptoms of more than one of these three forms. The most common mixed form includes spasticity and athetoid movements. Even among these mixed forms, individuals can range from very mild to completely incapacitated, Smith says.

Non-Mobility Considerations

Although CP does not directly cause respiratory problems, Miles says children with CP are prone to respiratory issues, especially with quadriplegic involvement and poor trunk control. "They don't have the ability to cough up all of that phlegm," she says. "So, when they do get a respiratory thing, it can sometimes become more serious for them than it would for somebody else."

For clients with feeding issues, including difficulty swallowing, aspirating on food is a problem and can result in secondary complications, such as pneumonia, Miles explains. Feeding tubes may be necessary.

Poor nutrition also is an issue for CP clients with feeding issues. "It's hard for them to eat and try to get all of those calories in," she says. "Sometimes they'll be on feeding tubes even if they can do some eating."

Clients with CP, especially those that are highly involved, may be unable to adjust their sleeping position, Miles says. As a result, many clients with CP may have trouble sleeping through the night.

"Due to muscle and motor involvement, positioning and communication skills can be very involved based on severity," Smith says. Severe symptoms may cause a person to remain in a permanent fetal position, which means communication skills may be nonexistent.

Still, be careful when assessing cognition and intelligence, Smith warns. "Experts note that it's important not to underestimate the cognition and intelligence of those with cerebral palsy, as it ranges from genius to mental retardation on the same scale as the general population. That is, there's no evidence that cerebral palsy impacts intelligence."

The Effects of Aging

According to Children's Hemiplegia and Stroke Association (CHASA), the symptoms of CP do change over time, though the condition is not progressive.

"We know folks with CP deteriorate," Paleg says. "We see osteopenia, loss of range of motion, and especially the kids that use walkers can lose this skill between ages 15 and 22, when they have their final growth spurt. Most adults with CP complain about pain and depression."

Smith says age can impact symptoms positively or negatively. "For example, a young child may be able to walk until a certain age, when a taller stature becomes a greater consequence due to his or her limited balance, at which point the effects of cerebral palsy are more profound," he says. "However, with therapy, some with cerebral palsy can gain increased function, where symptoms then have less effect in the long term."

Miles says spasticity plays an important role in function as children with CP age. "The kids who have spasticity, as their bones grow, their muscles aren't going to grow at the same rate because (of) that muscle tone," she says.
When these children go through a growth spurt, it's important for them to be involved in a therapy program or an intense at-home program to make sure the muscles are stretched. If that doesn't happen, these children will begin to look like they're doing worse, with symptoms like toe walking or walking with bent knees, Miles explains.

"They might find it harder and harder to walk because it takes them longer because their stride length is shorter," she says.

Also, when the muscles get too tight, the hips can get out of alignment, causing pull on the trunk muscles, which leads to scoliosis.

An important distinction between treating children and adults, according to Smith, is the type of intervention involved. For children, it's more intervention based, including physical therapy, speech therapy, intellectual development and surgery. Adults, on the other hand, require more occupational therapy, including improving quality of life, overall living skills and employment - although physical therapy also may continue.

Miles says it may be more difficult for teenagers and young adults with CP to get the therapy they need because they often do not present big functional changes. "But I think we have to remember if we don't do some of that intervention, some of those kids and young adults are going to lose some of their function," she says.

In fact, Miles often sees loss of function with teens she thought would walk into adulthood. A lot of these teens end up choosing wheelchairs simply because they find it more difficult to do things. Miles says this choice usually comes about because the teens don't have standers and intervention programs at home that can help them. She encourages therapists to educate families to make sure these teens do go back for therapy to maintain function.

"For these older young adults, we need to find ways for them to do fitness and workout so that they can maintain a healthy lifestyle," Miles says.

The Role of Assistive Technology

"There's a bell curve to the severity of cerebral palsy symptoms and the assistive technology needed for daily living," Smith says.
On the two ends of the spectrum are mild and severe cerebral palsy - both of which may not require assistive technology. The large portion of those in between, however, rely on wheelchairs, custom seating, communication devices and more.

"When it comes to cerebral palsy," Smith says, "there's such a wide variation in the consequence of symptoms - from affected posture to range of motion to effective coordination - that seating and mobility equipment is truly custom to each individual.

"Much of the assistive technologies used by those with cerebral palsy require adaptation, as the effects of symptoms are rarely identical from one user to the next," Smith continues. "For example, in order to operate a power wheelchair, based on the severity of cerebral palsy, the user may require a standard joystick control or a highly adapted head, lap or foot control."

As a result, assistive technologies are often modified for each user.
Clients with severe CP who are not incapacitated require more complex rehab. Smith says these technologies range from custom-molded seating to specialty drive controls to operate a power wheelchair.

Because clients with diplegia tend to be higher functioning, they may be able to walk unassisted or with an assistive device and only need a wheelchair for longer distances, Miles says.

In fact, those with diplegic involvement and good upper-extremity movement should be able to climb stairs and do activities of daily living, like dressing and toileting.On the other hand, quadriplegic involvement may limit the ability to walk without an assistive device. "Those kids tend to be more wheelchair users, (and) some of them (are) able to use a power chair to get around, depending on their cognition and their ability to use their upper extremities," Miles says. "Some of those children will be manual wheelchair users and might not be able to push their chair. (They) might need to be pushed around by a caregiver and don't really have the potential for power wheelchair mobility because they don't have the control with their arms."

Those with quadriplegic CP often require more help with seating and positioning as well, Miles says. "I think a lot of what makes a difference with how much positioning and aiding you have ... is if you have a lot of asymmetries," she says.

Miles says children with hypotonia tend to be more independent ambulators, though they may still need an assistive device. Children with mixed tone, if they have low tone in their trunk and high tone in their extremities, will probably need a wheelchair.

Scoliosis is a common problem among clients with spastic CP and is something to watch out for, especially with children who use power wheelchairs.

"I personally have seen (scoliosis) a lot more with the higher muscle tone kids and more so with the children who need to use a wheelchair for mobility, especially if you have somebody who drives a power wheelchair," Miles says. Drive controls are usually placed on the less affected side, Miles says, but then the child begins to use that side more and develops compensations because of the asymmetries.

A seating system can address these asymmetries, but Miles says that even when clinicians stay on top of seating, scoliosis still can develop.

One of the major issues with scoliosis is that it can compromise respiration. "It's an important thing to watch because of the respiration and how much that's going to impact their ability to participate, be comfortable and fight off infections as they get older if they are losing some of that chest expansion ability," Miles says.

Standers are a good way to alleviate some of the issues CP clients may have regarding circulation, respiration and digestion, Miles says.

Communication also may be a problem with CP clients. Miles says communication can be related to cognition or to muscle coordination. Those will severe brain injuries may lack the cognition to use augmentative communication devices, whereas a non-verbal client with cognitive ability can make a choice to communicate.

For nonverbal clients that do want to communicate, Miles says, "I think we're being more creative with where we're placing communication devices. We can use a foot. We can use a head and all different things instead of just using their hands to communicate if they're not verbal."